The prognostic value of HFA-PEFF score in connective tissue disease-associated PAH: evidence from a cohort study

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BACKGROUND: Connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) patients with left heart dysfunction may have worse prognosis. This study was to investigate the prognostic value of HFA-PEFF score in connective tissue disease-associated PAH. METHODS: This single-center retrospective cohort study enrolled 147 CTD-PAH patients diagnosed via right heart catheterization (RHC), divided into two groups based on their HFA-PEFF scores: < 5 (n = 74) and ≥ 5 (n = 73). The clinical characteristics were compared between the two groups. The primary end point was all-cause mortality, and the secondary end point was clinical worsening events. Survival was analyzed using Kaplan-Meier analysis and Cox proportional hazards models. RESULTS: Compared to the HFA-PEFF score < 5 group, the ≥ 5 group exhibited significantly higher levels of N-terminal pro-brain natriuretic peptide (NT-proBNP), a greater proportion of WHO functional class III-IV, shorter 6-min walk distance (6MWD), larger right ventricular (RV) volume, worse RV function, smaller left ventricular (LV) volume, and higher native T1 values. An HFA-PEFF score ≥ 5 was a predictor for all-cause mortality in CTD-PAH (HR 5.022, P = 0.020) and for clinical worsening events (HR 2.670, P = 0.020). At follow-up, 17.9% of CTD-PAH had an HFA-PEFF score ≥ 5. Patients with follow-up HFA-PEFF scores ≥ 5 had a significantly lower event-free survival rate (P < 0.001). CONCLUSION: An HFA-PEFF score ≥ 5 was associated with all-cause mortality and clinical worsening events in CTD-PAH patients. TRIAL REGISTRATION: NCT05980728. Connective Tissue Disease Patients With Pulmonary Hypertension. Oct 17, 2023.

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